Primary central nervous system lymphoma presenting as a single choroidal lesion mimicking metastasis:A case report

INTRODUCTION

Primary intraocular lymphoma (PIOL) is a rare subset of primary central nervous system lymphoma (PCNSL) and includes vitreoretinal,choroidal,and iridal lymphomas [1].Among PIOLs,primary choroidal lymphoma is a very rare disease,and only a small number of cases have been reported in the literature.Previously reported primary choroidal lymphomas were usually low-grade B-cell lymphomas,which rarely progressed to the central nervous system[2,3].Herein,we report a very rare case of primary choroidal lymphoma diagnosed as diffuse large B-cell lymphoma (DLBL),which initially presented as a unilateral choroidal lesion mimicking metastasis.

CASE PRESENTATION

Chief complaints

A 78-year-old male came to the hospital presenting with a diminution of vision in his right eye.

History of present illness

His blurred vision had been aggravated for several weeks.

History of past illness

The patient had no relevant medical history,such as that of diabetes mellitus or hypertension.In the past,the patient had undergone short-term treatment for occupational pneumoconiosis resulting from his work as a coal miner for＞10 years.

Personal and family history

No special history of personal and family.

Physical examination

After completing six cycles of HD-MTX-based chemotherapy,the patient’s neurological symptoms,such as right-sided weakness,dysarthria,and blurred vision,markedly improved.The abnormal,contrast-enhancing mass in the left cerebellum disappeared on brain MRI (Figure 2C),and the creamy yellow,subretinal infiltrates were markedly decreased (Figure 1C);these findings were compatible with complete remission.Maintenance therapy was recommended,but the patient and caregiver refused the treatment due to concerns about the side effects of additional chemotherapy and radiotherapy.The patient and his main caregiver were seriously concerned about the risk of cognitive decline that can be caused by additional treatments.Complete remission was maintained for approximately 17 mo without the maintenance chemotherapy.

The maiden50 did as he told her, and then the wolf breathed over the flowery grave, and, lo and behold51! the Prince lay there sleeping as peacefully as a child

Laboratory examinations

Lactate dehydrogenase was moderately elevated at 387 U/L (normal range:119-229 U/L).

Imaging examinations

To identify the primary tumor,the patient was referred to the Department of Hematooncology at our institute.Brain magnetic resonance imaging (MRI) (Figure 2A) and computed tomography (CT) of the neck,chest,and abdomen-pelvis showed no malignant lesions.

But she shook her head silently. As they pressed her further with questions, she threw them the golden chain from her neck.36 But they did not leave off, and she threw them her girdle,37 and when this was no use, her garters,38 and then her dress.39 The huntsmen would not leave her alone, but climbed the tree, lifted the maiden down, and led her to the King. The King asked, Who are you? What are you doing up that tree?

FINAL DIAGNOSIS

Primary choroidal lymphomas are mainly low grade B-cell lymphomas such as extranodal marginal zone B-cell lymphoma,are usually unilateral,and typically do not progress to the central nervous system parenchyma.Secondary choroidal lymphoma is characterized by the presence of previously known cancer or concurrent systemic lymphomas at the initial ocular presentation.

TREATMENT

The patient received high-dose methotrexate (HD-MTX)-based chemotherapy,which comprised methotrexate (3.5 g/m),vincristine (1.4 mg/m,capped at 2 mg),and prednisolone (100 mg/d),with the addition of procarbazine (100 mg/m) in the first,third,and fifth cycles.Intrathecal injection was not given in this patient because there was no evidence of lymphoma involvement in the CSF test before and after the first cycle of HD-MTX based chemotherapy.

OUTCOME AND FOLLOW-UP

An ophthalmological examination revealed exudative subretinal detachment with a choroidal lesion suspicious of metastasis in the right eye (Figure 1A).

DISCUSSION

In contrast to primary choroidal lymphomas,secondary choroidal lymphomas are more likely to demonstrate bilateral involvement and preexistent lymphomas.More than half of secondary choroidal lymphomas have been confirmed as high-grade B-cell lymphomas,such as DLBL[2,8].

Primary intraocular lymphoma is considered a subset of PCNSL,and it progresses to the central nervous system in 15%-25% of PCNSL cases[3].Primary vitreoretinal lymphoma is the most common intraocular lymphoma,followed by uveal lymphoma.

Hardly were the words well out of his mouth, when castle, cupboard, and all vanished, and there they were sitting in their poor little fishing hut once more

Unlike the previously reported cases of primary choroidal lymphoma,this case was characterized by the pathological findings of DLBL and disease progression to the brain parenchyma within a few months.In most of the previous cases of primary choroidal lymphoma,management involved local treatment or observation,whereas in our case,HD-MTX-based chemotherapy was administered,and the treatment response was complete remission.

Four months later,the patient revisited the clinic because of dysarthria,headache,and right-sided weakness.No apparent aggravation of visual acuity was observed.The same findings of ophthalmoscopy as at the first visit were confirmed (Figure 1B).Brain MRI showed a single,irregular,contrast-enhancing mass in the left cerebellum (Figure 2B).The patient underwent a stereotactic biopsy of the mass and was diagnosed with DLBL (Figure 3).Further imaging,including CT and positron emission tomography/CT,revealed no systemic involvement.There was no evidence of lymphoma involvement in the cerebrospinal fluid and bone marrow.

Intraocular lymphoma is extremely rare and accounts for approximately 1.86% of intraocular malignancies[4].Intraocular lymphoma is a heterogeneous group of malignancies located in different tissues,including the vitreous,retina,choroid,ciliary body,and iris,within the eye.It also refers to forms of primary or secondary to central nervous system lymphoma or disseminated systemic disease[3,5].

Choroidal lymphoma is a subset of uveal lymphoma.It can be subdivided into primary and secondary lymphoma based on the presence of systemic lymphoma at the time of ocular presentation.Primary choroidal lymphomas are defined as the absence of prior systemic lymphomas or concurrent extraocular lymphomas [6].Several studies have reported clinical differences between primary and secondary choroidal lymphomas[2,7].

The man s heart grew heavy, and he would not go. He said to himself, It is not right, and yet he went. And when he came to the sea the water was quite purple and dark-blue, and grey and thick, and no longer so green and yellow, but it was still quiet. And he stood there and said --

So Tsarevitch Ivan mounted the Gray Wolf and the Tsarevna rode on the Horse with the Golden Mane, and at length they came to the forest where the Wolf had devoured Tsarevitch Ivan s horse.

Observe a child; any one will do. You will see that not a day passes in which he does not find something or other to make him happy, though he may be in tears the next moment. Then look at a man; any one of us will do. You will notice that weeks and months can pass in which day is greeted with nothing more than resignation, and endure with every polite indifference1. Indeed, most men are as miserable2 as sinners, though they are too bored to sin-perhaps their sin is their indifference. But it is true that they so seldom smile that when they do we do not recognize their face, so distorted is it from the fixed3 mask we take for granted. And even then a man can not smile like a child, for a child smiles with his eyes, whereas a man smiles with his lips alone. It is not a smile; but a grin; something to do with humor, but little to do with happiness. And then, as anyone can see, there is a point (but who can define that point?) when a man becomes an old man, and then he will smile again.

CONCLUSION

Primary choroidal lymphoma is generally known to have a benign clinical course without systemic involvement.We reported a rare case of primary choroidal lymphoma diagnosed as DLBL,characterized by an aggressive clinical course that progressed to the brain parenchyma within a few months.

The authors thank the Department of Radiology,Kangwon National University Hospital,Kangwon National University School of Medicine.